Recombinant Human M6PR (cation dependent) protein - BSA and Azide free (His tag N-Terminus)

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M6PR or mannose-6-phosphate receptor (cation dependent) is a protein involved in the transport of lysosomal enzymes from the Golgi apparatus to the lysosome. Alternate names for M6PR include CD-MPR and MPR46 reflecting its function and molecular weight of approximately 46 kDa. M6PR is expressed in various tissues but primarily found in the Golgi apparatus where it engages with lysosomal enzymes. This receptor is an important element in the delivery system that ensures lysosomal enzymes reach their intended cellular destination.

Biological function summary

M6PR acts as a sorting receptor by binding mannose-6-phosphate-tagged enzymes in the Golgi. It is involved in a complex trafficking mechanism that involves clathrin-coated vesicles. The receptor recognizes and binds to its ligands in the Golgi directing them to endosomes and eventually fusing with lysosomes. M6PR plays a critical role in maintaining lysosome functionality by regulating enzyme availability which is essential for cellular waste degradation and resource recycling.

Pathways

The protein M6PR participates in the lysosomal enzyme targeting pathway that ensures proper enzyme sorting and delivery within cells. This pathway overlaps with the Golgi-to-endosome transport vesicle biogenesis involving proteins like clathrin and adaptin. M6PR interacts with proteins in the sorting pathway such as GGA adaptors and Hsc70 which assist in the recognition and transport processes necessary for lysosome integrity and function.

The malfunction of M6PR links to disorders like I-cell disease and mucolipidosis II characterized by defective lysosomal enzyme targeting. Impaired M6PR function can lead to the accumulation of undigested substrates within the lysosome causing cellular dysfunction. The receptor's failure can also indirectly affect protein interactions in diseases like mucolipidosis where relationships with proteins such as IGF2R can get altered further influencing cellular pathways and leading to various pathological conditions.